Defective intracellular activity of GDP- d-mannose-4,6-dehydratase in leukocyte adhesion deficiency type II syndrome

Leukocyte adhesion deficiency type II (LAD II) is a rare genetic disease characterized by severe immunodeficiency which is related to defective expression in leukocytes of sialyl-Lewis X (SLeX), a fucosylated ligand for endothelial selectins. The molecular basis of LAD II is still unknown, but has b...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	FEBS letters 1998-06, Vol.429 (3), p.274-278
Hauptverfasser:	Sturla, Laura, Etzioni, Amos, Bisso, Angela, Zanardi, Davide, De Flora, Giovanni, Silengo, Lorenzo, De Flora, Antonio, Tonetti, Michela
Format:	Artikel
Sprache:	eng
Schlagworte:	AEBSF, 4-(2-aminoethyl)-benzenesulfonyl fluoride Cloning, Molecular EBV, Epstein-Barr virus ECL, enhanced chemiluminescence FCS, fetal calf serum GDP- d-mannose-4,6-dehydratase GMD, GDP-d-mannose-4,6-dehydratase Guanosine Diphosphate Fucose - biosynthesis Guanosine Diphosphate Mannose - metabolism Humans Hydro-Lyases - genetics Hydro-Lyases - metabolism IPTG, isopropyl-β-thiogalactopyranoside l-Fucose LAD II, leukocyte adhesion deficiency type II Leukocyte adhesion deficiency type II Leukocyte-Adhesion Deficiency Syndrome - enzymology Leukocyte-Adhesion Deficiency Syndrome - genetics Lymphocyte Male Oligosaccharides - biosynthesis PHA-M, phytohemagglutinin M PMSF, phenylmethylsulfonyl fluoride Recombinant Proteins - metabolism RNA, Messenger - genetics SDS-PAGE, sodium dodecylsulfate-polyacrylamide gel electrophoresis Sequence Analysis, DNA Sialyl-Lewis X SLeX, sialyl-Lewis X
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Schreiben Sie den ersten Kommentar!